Association between routine blood biomarkers, lung phenotypes and disease severity in patients with alpha-1 antitrypsin Pi*SZ deficiency from the EARCO Registry

There is limited information on prognostic blood biomarkers in alpha-1-antitrypsin deficiency (AATD). Our objetive was to identify possible associations between serum biomarkers, lung phenotypes and the severity of lung disease in patients with Pi*SZ genotype Retrospective study of patients with AATD Pi*SZ from the database of the international EARCO (European Alpha-1 Research Collaboration) registry. Serum biomarkers and forced expiratory volume in the first second (FEV1%) were collected. Neutrophil/lymphocyte ratio (NLR), eosinophil/lymphocyte ratio (ELR), and platelet/lymphocyte ratio (PLR) were calculated. Correlations between biomarkers and lung function were investigated 373 patients with Pi*SZ AATD were included. 49.3% were men, with a mean age of 52.2 (SD:16.2) years and a mean FEV1 of 92.7% (25). A correlation was observed between a lower FEV1% and higher levels of AAT, GGT, FIB-4, hemoglobin, leukocytes, neutrophils and NLR. Pi*SZ patients with emphysema were older, with more comorbidities and exacerbations than those who had asthma or bronchiectasis. Patients with emphysema had significantly higher levels of AAT, AST, GGT, leukocytes and neutrophils and lower levels of hemoglobin and FIB-4. Individuals with bronchiectasis had higher levels of eosinophils and ELR and those with asthma only had lower FIB-4 levels Impaired FEV1% was associated with higher levels of inflammatory and liver biomarkers. Emphysema was associated with increased liver enzymes and inflammation, a finding not present in patients with asthma or bronchiectasis. The Pi*SZ patients with emphysema were older, with more comorbities and exacerbations